Hernia diafragmática congénita de aparición tardía: la importancia de la clínica / Tardive appearing congenital diaphragmatic hernia: the importance of the symptoms

El diagnóstico de hernia diafragmática es raro después del período neonatal. Presentamos a un lactante de 8 meses con decaimiento y diagnóstico final de hernia diafragmática, ya que se trata de una presentación atípica, en una edad poco común y que presentó una evolución tórpida, a fin de difundir el conocimiento de esta entidad y sus posibles presentaciones a la comunidad científica (AU)

Diaphragmatic hernia diagnosis after neonatal period is a rare issue. We show a 8 month-baby with decay and final diagnosis of late-onset diaphragmatic hernia, because it is an atypical presentation, an uncommon age, and a torpid evolution, thus, it would be interesting to spread this subject to scientific community (AU)

Tratamiento antimicrobiano parenteral domiciliario: análisis prospectivo de los últimos 12 años / Domiciliary parenteral antibiotic therapy: A prospective analysis of the last 12 years

OBJETIVO: Clásicamente la administración de tratamientos intravenosos se ha asociado a la necesidad de ingreso hospitalario. A partir de la formación de unidades de hospitalización a domicilio (UHD) se han extendido e incrementado el número de tratamientos antimicrobianos administrados por vía parenteral en pacientes pediátricos en su domicilio. El objetivo de este artículo es exponer la experiencia en tratamientos parenterales en domicilio en una UHD pediátrica durante un periodo de 12 años. Pacientes y método: Se realiza un estudio descriptivo, prospectivo. Se incluyeron en el estudio a todos los pacientes atendidos en nuestra UHD por enfermedad subsidiaria de tratamiento parenteral desde enero de 2000 hasta diciembre de 2012. RESULTADOS: Se han instaurado 163 tratamientos parenterales en domicilio. La edad media fue de 11,1 años. Por sexos, 40% mujeres y 60% varones. Las infecciones tratadas han sido, en su mayoría, sobreinfecciones respiratorias (76%), infecciones del catéter central (9,2%) e infecciones del tracto urinario (5,5%). El antibiótico más utilizado ha sido la amikacina. El 96,9% de los tratamientos fue intravenoso. La complicación más frecuente ha sido la extravasación o pérdida de vía (7,4%). Se completó el tratamiento en domicilio en un 90,2% de los casos. CONCLUSIONES: La administración de tratamientos parenterales en domicilio es una opción asistencial buena y segura en pacientes pediátricos

OBJETIVES: Parenteral antibiotic treatment has been classically developed in hospitals and is considered as a hospital procedure. The development of Hospital at Home Units (HHU) has led to an increase in outpatient parenteral antibiotic therapy (OPAT) in paediatrics patients. The objective of this study is to describe our experience, as an HHU integrated within a Paediatric Department, in home antimicrobial therapy over a period of 12 years. PATIENTS AND METHODS: This prospective and descriptive study included every patient with a disease requiring parenteral antimicrobial therapy who was admitted to our HHU from January 2000 to December 2012. RESULTS: During the study there were 163 cases on OPAT. The mean age of the patients was 11.1 years, and the sample group was comprised of 33 males and 22 feamales. The main sources of the treated infections were respiratory tract (76%), catheter-related bloodstream (9.2%), and urinary tract infections (5.5%). Amikacin was the most widely used antibiotic. Almost all treatments (96.6%) were via an intravenous route. Catheter-associated complications were more common than drug-associated complications. Successful at-home treatment was observed in 90.2% of cases. CONCLUSIONS: OPAT is a good and safe alternative in many paediatric diseases

[Domiciliary parenteral antibiotic therapy: a prospective analysis of the last 12 years]. / Tratamiento antimicrobiano parenteral domiciliario: análisis prospectivo de los últimos 12 años.

OBJETIVES: Parenteral antibiotic treatment has been classically developed in hospitals and is considered as a hospital procedure. The development of Hospital at Home Units (HHU) has led to an increase in outpatient parenteral antibiotic therapy (OPAT) in paediatrics patients. The objective of this study is to describe our experience, as an HHU integrated within a Paediatric Department, in home antimicrobial therapy over a period of 12 years. PATIENTS AND METHODS: This prospective and descriptive study included every patient with a disease requiring parenteral antimicrobial therapy who was admitted to our HHU from January 2000 to December 2012. RESULTS: During the study there were 163 cases on OPAT. The mean age of the patients was 11.1 years, and the sample group was comprised of 33 males and 22 feamales. The main sources of the treated infections were respiratory tract (76%), catheter-related bloodstream (9.2%), and urinary tract infections (5.5%). Amikacin was the most widely used antibiotic. Almost all treatments (96.6%) were via an intravenous route. Catheter-associated complications were more common than drug-associated complications. Successful at-home treatment was observed in 90.2% of cases. CONCLUSIONS: OPAT is a good and safe alternative in many paediatric diseases.

Tuberculosis endobronquial que simula un enfisema lobar congénito / Endobronchial tuberculosis simulating congenital lobular emphysema

No disponible

[Prevalence of metabolic syndrome and its components in obese children and adolescents]. / Prevalencia del síndrome metabólico y sus componentes en niños y adolescentes con obesidad.

OBJECTIVE: The aim of our study was to assess the prevalence of metabolic syndrome and other metabolic features in obese children. METHODS: We studied 97 obese children and adolescents (body mass index > or = 95th percentile) aged between 6 and 14 years old. All children underwent an oral glucose tolerance test. The diagnoses of fasting impaired glucose, impaired glucose tolerance and type 2 diabetes were defined according de American Diabetes Association criteria. Diagnosis of metabolic syndrome was defined according de National Cholesterol Education Program-Adult Treatment Panel (NCEP-ATP III) criteria adapted for children. RESULTS: Metabolic syndrome was found in 18.6 % of the children, with a higher rate among puberal children (26.2 %) than among prepuberal children (12.7 %). There were no significant differences by sex. The prevalence of each of the components was 10.3 % for abnormal glucose homeostasis (8.2 % in impaired fasting glucose, 5.4 % in impaired glucose tolerance, 1.1 % in type 2 diabetes), 97.9 % for abdominal obesity, 16.5 % for high triglyceride level, 15.5 % for low levels of high-density lipoprotein cholesterol, and 45.4 % for high blood pressure (40.2 % for systolic pressure and 22.7 % for diastolic pressure). Insulin resistance (HOMA-R > or = 3.8) was found in 45.4 % of the children, with a higher rate among children with metabolic syndrome (77.8 %). CONCLUSIONS: Nearly 20 % of the obese children studied met the criteria for metabolic syndrome, a constellation of metabolic derangements associated with obesity. Insulin resistance was very common among children with obesity and metabolic syndrome.

Prevalencia del síndrome metabólico y sus componentes en niños y adolescentes con obesidad / Prevalence of metabolic syndrome and its components in obese children and adolescents

Objetivo El propósito principal de este estudio fue establecer la prevalencia del síndrome metabólico (SM) y sus componentes en niños con obesidad. Métodos Se estudiaron 97 niños y adolescentes con obesidad (IMC ≥ P95) entre 6-14 años de edad. Cada sujeto fue sometido a una sobrecarga oral con glucosa. Para el diagnóstico de intolerancia a la glucosa, alteración de la glucemia en ayunas y diabetes mellitus se siguieron las recomendaciones de la Asociación Americana de Diabetes (ADA). La definición de SM fue adaptada de la recomendada por el National Cholesterol Education Program-Adult Treatment Panel III (NCEP-ATP III). Resultados El SM se encontró en el 18,6 % de los sujetos con una frecuencia mayor en sujetos puberales (26,2 %) que en prepuberales (12,7 %). No hubo diferencias entre sexos. La prevalencia de cada uno de los componentes del mismo fue del 10,3 % para la alteración del metabolismo de la glucosa (8,2 % para la alteración de la glucemia en ayunas; 5,4 % para la intolerancia a la glucosa, y 1,1 % para la diabetes tipo 2); 97,9 % para la obesidad central; 16,5 % para la hipertrigliceridemia; 15,5 % para el colesterol de lipoproteínas de alta densidad (HDL-c) bajo y 45,4 % para la hipertensión arterial (40,2 % para la sistólica y 22,7 % para la diastólica). El 45,4 % de los sujetos estudiados presentó insulinorresistencia (HOMA-R ≥ 3,8), que se elevó hasta el 77,8 % en sujetos con SM. Conclusiones Casi el 20 % de los niños estudiados cumplía criterios de síndrome metabólico, una constelación de anomalías metabólicas relacionadas con la obesidad. La insulinorresistencia fue muy frecuente en niños con obesidad y SM

Objetive The aim of our study was to assess the prevalence of metabolic syndrome and other metabolic features in obese children. Methods We studied 97 obese children and adolescents (body mass index ≥ 95th percentile) aged between 6 and 14 years old. All children underwent an oral glucose tolerance test. The diagnoses of fasting impaired glucose, impaired glucose tolerance and type 2 diabetes were defined according de American Diabetes Association criteria. Diagnosis of metabolic syndrome was defined according de National Cholesterol Education Program-Adult Treatment Panel (NCEP-ATP III) criteria adapted for children. Results Metabolic syndrome was found in 18.6 % of the children, with a higher rate among puberal children (26.2 %) than among prepuberal children (12.7 %). There were no significant differences by sex. The prevalence of each of the components was 10.3 % for abnormal glucose homeostasis (8.2 % in impaired fasting glucose, 5.4 % in impaired glucose tolerance, 1.1 % in type 2 diabetes), 97.9 % for abdominal obesity, 16.5 % for high triglyceride level, 15.5 % for low levels of high-density lipoprotein cholesterol, and 45.4 % for high blood pressure (40.2 % for systolic pressure and 22.7 % for diastolic pressure). Insulin resistance (HOMA-R ≥ 3.8) was found in 45.4 % of the children, with a higher rate among children with metabolic syndrome (77.8 %). Conclusions Nearly 20 % of the obese children studied met the criteria for metabolic syndrome, a constellation of metabolic derangements associated with obesity. Insulin resistance was very common among children with obesity and metabolic syndrome

[Major histocompatibility complex class II deficiency]. / Déficit de expresión de moléculas de clase II del complejo mayor de histocompatibilidad.

Major histocompatibility complex class II deficiency is an autosomal recessive primary combined immunodeficiency. The prevalence of this deficiency is highest in Mediterranean areas, especially north Africa. Early diagnosis is essential due to high mortality in the first 2 years of life and the possibility of bone marrow transplantation. We report four cases of major histocompatibility complex class II deficiency and describe their epidemiologic and clinical characteristics, diagnostic tests, treatment and outcome.

Déficit de expresión de moléculas de clase II del complejo mayor de histocompatibilidad / Major histocompatibility complex class II deficiency

El déficit de expresión de moléculas de clase II del complejo mayor de histocompatibilidad es una inmunodeficiencia primaria combinada de herencia autosómica recesiva. Presenta mayor prevalencia en los países mediterráneos, sobre todo en el norte de África. La precocidad en el diagnóstico es vital, dada su elevada letalidad en los primeros 2 años de vida, así como su potencial tratamiento mediante trasplante de progenitores hematopoyéticos. Se presenta una revisión de 4 casos mediante la descripción de las características epidemiológicas y clínicas, las pruebas diagnósticas, el abordaje terapéutico y la posterior evolución

Major histocompatibility complex class II deficiency is an autosomal recessive primary combined immunodeficiency. The prevalence of this deficiency is highest in Mediterranean areas, especially north Africa. Early diagnosis is essential due to high mortality in the first 2 years of life and the possibility of bone marrow transplantation. We report four cases of major histocompatibility complex class II deficiency and describe their epidemiologic and clinical characteristics, diagnostic tests, treatment and outcome

Infección perinatal por VIH-1 no esperada / Unexpected perinatal HIV-1 infection

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[Drowning in pediatric patients]. / Ahogamientos y casi ahogamientos en niños.

OBJECTIVE: Immersion accidents are still an important cause of morbidity and mortality in children. We performed a retrospective study to identify the prognostic factors associated with outcome in children who experience near-drowning, which could serve to guide decision-making. Our data were compared with other published data. PATIENTS AND METHODS: The medical records of children treated for near-drowning in our hospital from January 1995 to April 2003 were reviewed. The data analyzed referred to the patient, the accident, the patient's clinical status in the emergency unit, the unit to which the patient was admitted, and outcome. RESULTS: Sixty-two patients were included. Of these, outcome was bad in 12 (death in seven and irreversible sequelae in five). Statistically significant predictors of bad prognosis were age > or = 4 years, female sex, immersion time > or = 5 min, cyanosis in the emergency room, cardiac arrest, apnea or severe distress, hypothermia (core temperature < 35 degrees C), metabolic acidosis (pH < or = 7.10) and neurologic damage (Glasgow coma Scale score 3; Conn C; nonreactive and mydriatic pupils) on arrival at the hospital. CONCLUSIONS: Outcome is closely related to the patient's clinical status on arrival at the hospital. Although data that can serve as a guide to the final outcome of the nearly-drowned patient are available, early models to predict the final clinical results of each case, which could be used to guide initial resuscitation and subsequent treatment, are lacking.

Ahogamientos y casi ahogamientos en niños / Drowning in pediatric patients

Objetivo: Los accidentes por sumersión siguen causando importante morbimortalidad en niños. Se realiza un estudio retrospectivo para identificar los factores pronósticos asociados a la evolución clínica de niños con accidente por sumergimiento, que puedan servir de ayuda para tomar decisiones terapéuticas, comparándolos con otros datos publicados. Pacientes y métodos: Estudio retrospectivo mediante revisión de las historias clínicas de los casos atendidos por accidente por sumergimiento entre enero de 1995 y abril de 2003. Los datos analizados se refieren al paciente, el accidente, situación clínica en Urgencias y unidades donde ingresan en el hospital, valorando la evolución final de cada caso. Resultados: Se han incluido 62 pacientes, presentando mala evolución 12 (7 fallecimientos y 5 secuelas permanentes). Han resultado significativos, como datos de mal pronóstico, edad igual o superior a 4 años, sexo femenino, tiempo inmersión 5 min o más y, a la llegada al hospital: cianosis inicial que persistía en Urgencias; parada cardíaca, apnea o distrés grave, hipotermia (temperatura < 35 °C), acidosis metabólica (pH < 7,10) y afección neurológica (Glasgow 3; Conn C; pupilas midriáticas y arreactivas). Conclusiones: La evolución final del paciente está estrechamente relacionada con la situación clínica a su llegada al hospital. Aunque disponemos de datos orientativos sobre la evolución posterior del paciente casi ahogado, se carece de modelos predictivos precoces que permitan vaticinar la evolución del paciente y, por lo tanto, orientar en la actitud a tomar en las medidas iniciales de reanimación y mantenimiento posterior

Objective: Immersion accidents are still an important cause of morbidity and mortality in children. We performed a retrospective study to identify the prognostic factors associated with outcome in children who experience neardrowning, which could serve to guide decision-making. Our data were compared with other published data. Patients and methods: The medical records of children treated for near-drowning in our hospital from January 1995 to April 2003 were reviewed. The data analyzed referred to the patient, the accident, the patient’s clinical status in the emergency unit, the unit to which the patient was admitted, and outcome. Results: Sixty-two patients were included. Of these, outcome was bad in 12 (death in seven and irreversible sequelae in five). Statistically significant predictors of bad prognosis were age >= 4 years, female sex, immersion time >= 5 min, cyanosis in the emergency room, cardiac arrest, apnea or severe distress, hypothermia (core temperature < 35 °C), metabolic acidosis (pH <= 7.10) and neurologic damage (Glasgow coma Scale score 3; Conn C; nonreactive and mydriatic pupils) on arrival at the hospital. Conclusions: Outcome is closely related to the patient’s clinical status on arrival at the hospital. Although data that can serve as a guide to the final outcome of the nearly-drowned patient are available, early models to predict the final clinical results of each case, which could be used to guide initial resuscitation and subsequent treatment, are lacking

[Deep venous thrombosis: an early manifestation of Behçet's disease in childhood]. / Trombosis venosa profunda como manifestación precoz de la enfermedad de Behçet en la infancia.

Behcet's disease is a multisystemic vasculitis of unknown origin whose classical triad is oral and genital ulcers and uveitis. Deep venous thrombosis may sometimes be the first manifestation of this disease but is very rare in children. We report the case of a 14-year-old boy with deep venous thrombosis of the leg and a history of severe aphthous gingivostomatitis, perianal ulcers, Henoch-Schonlein purpura, ecchymotic lesions on the lower limbs and intermittent swelling of the left ankle. Complementary tests ruled out the main causes of thrombosis in children. HLA-B5 and B-51 study proved positive, which, together with the patient's history, suggested a diagnosis of Behcet's disease. This disease usually presents around the third decade of life and is very unusual in children. This case is exceptional because thrombosis as an early manifestation of Behcet's disease is very rare in children and because it led to the diagnosis of Behcet's disease in our patient.

Trombosis venosa profunda como manifestación precoz de la enfermedad de Behçet en la infancia / Deep venous thrombosis: an early manifestation of Behçet´s disease in childhood

La enfermedad de Behçet es una vasculitis multisistémica de origen desconocido cuya tríada clásica son aftas orales, úlceras genitales y uveítis. En ocasiones, la trombosis venosa profunda puede ser la primera manifestación, pero es muy rara en niños. Se expone el caso de un varón de 14 años con trombosis venosa profunda del miembro inferior, con antecedentes de gingivostomatitis aftosas graves, úlceras perianales, púrpura de Schönlein-Henoch, lesiones equimóticas en miembros inferiores y tumefacción intermitente del tobillo izquierdo. Mediante pruebas complementarias se descartaron las principales causas de trombosis en niños. La positividad de los antígenos de histocompatibilidad B5 (HLA-B5) y B51, junto con las múltiples manifestaciones previas del paciente llevaron al diagnóstico de la enfermedad de Behçet. Esta se presenta generalmente en torno a la tercera década de la vida, y es poco frecuente en niños. Lo particular de este caso es que la trombosis como manifestación precoz en niños es excepcional y permitió diagnosticar la enfermedad (AU)

Anemia intensa en la primera infancia / Severe anemia in early childhood

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[Influence of energy expenditure on childhood obesity]. / Influencia del gasto energético en la obesidad infantil.

OBJECTIVES: To study differences in resting energy expenditure (REE) according to its determining factors (sex, weight, body fat mass, lean body mass) and in the oxidation of energy substrates in obese and non-obese children. PATIENTS AND METHODS: We studied 71 children (39 obese and 32 non-obese) aged from 4.1 to 13.6 years. The male/female ratio was 34/37. Energy expenditure (EE) was measured by using open circuit indirect calorimetry. The oxidation of energy substrates was calculated from oxygen consumption, carbon dioxide production, and urinary nitrogen excretion from urea. Body composition was determined by anthropometry. RESULTS: REE (kcal/day), adjusted for anthropometric parameters and body composition, was higher in boys than in girls. The absolute REE was significantly higher in obese than in non-obese children (1512.82 6 234.47 vs 1172.59 6 190.20) and was higher or the same when adjusted for its determinants. Compared with the non-obese group, the obese group presented a significantly higher percentage of fat oxidation (57.15 6 10.68 vs 51.08 6 13.61, p 5 0.04), a lower percentage of carbohydrate oxidation (30.10 6 9.85 vs 36.34 6 13.61, p 5 0.03) and a lower respiratory quotient (0.79 6 0.03 vs 0.82 6 0.04, p 5 0.02). No differences were found between male and female subjects in the percentages of carbohydrate, fat, and protein oxidation. CONCLUSIONS: We obtained the followings conclusions: a) When adjusted for anthropometric measurements and body composition, REE was significantly higher in boys than in girls; b) REE was higher in obese than in non-obese children, after adjustment for lean body mass; and c) Compared with the control group, obese children presented a higher percentage of fat oxidation, a lower percentage of carbohydrate oxidation, and a lower respiratory quotient.